Given that LARGE1 (acting as a bifunctional glycosyltransferase with both alpha-1,3-xylosyltransferase and beta-1,3-glucuronyltransferase activities) is localized to the ER-Golgi network and other ER-Golgi resident proteins were already unveiled as critical factors for motor neuron survival in SMA [5] and other motor neuron diseases such as ALS [10], were selected this protein as promising candidate for further analyses. Here, B3GAT3 is linked to amyotrophic lateral sclerosis.